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  Proteomics of Cytochrome c Oxidase-Negative versus -Positive Muscle Fiber Sections in Mitochondrial Myopathy

Murgia, M., Tan, J., Geyer, P. E., Doll, S., Mann, M., & Klopstock, T. (2019). Proteomics of Cytochrome c Oxidase-Negative versus -Positive Muscle Fiber Sections in Mitochondrial Myopathy. CELL REPORTS, 29(12), 3825-3834. doi:10.1016/j.celrep.2019.11.055.

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 Creators:
Murgia, Marta1, Author              
Tan, Jing2, Author
Geyer, Philipp E.1, Author              
Doll, Sophia1, Author              
Mann, Matthias1, Author              
Klopstock, Thomas2, Author
Affiliations:
1Mann, Matthias / Proteomics and Signal Transduction, Max Planck Institute of Biochemistry, Max Planck Society, ou_1565159              
2external, ou_persistent22              

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Free keywords: STOMATIN-LIKE PROTEIN-2; COMPUTATIONAL PLATFORM; REVEALS; DISEASE; IDENTIFICATION; BIOGENESIS; NUCLEAR; COMPLEX; CANCER; RATES
 Abstract: The mosaic distribution of cytochrome c oxidase(+) (COX+) and COX - muscle fibers in mitochondrial disorders allows the sampling of fibers with compensated and decompensated mitochondrial function from the same individual. We apply laser capture microdissection to excise individual COX+ and COX- fibers from the biopsies of mitochondrial myopathy patients. Using mass spectrometry-based proteomics, we quantify >4,000 proteins per patient. While COX+ fibers show a higher expression of respiratory chain components, COX- fibers display protean adaptive responses, including upregulation of mitochondrial ribosomes, translation proteins, and chaperones. Upregulated proteins include C1QBP, required for mitoribosome formation and protein synthesis, and STOML2, which organizes cardiolipin-enriched microdomains and the assembly of respiratory supercomplexes. Factoring in fast/slow fiber type, COX (-) slow fibers show a compensatory upregulation of beta-oxidation, the AAA(+) protease AFG3L1, and the OPA1-dependent cristae remodeling program. These findings reveal compensatory mechanisms in muscle fibers struggling with energy shortage and metabolic stress.

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Language(s): eng - English
 Dates: 2019
 Publication Status: Published in print
 Pages: 14
 Publishing info: -
 Table of Contents: -
 Rev. Type: Peer
 Degree: -

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Title: CELL REPORTS
Source Genre: Journal
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Publ. Info: 50 HAMPSHIRE ST, FLOOR 5, CAMBRIDGE, MA 02139 USA : CELL PRESS
Pages: - Volume / Issue: 29 (12) Sequence Number: - Start / End Page: 3825 - 3834 Identifier: ISSN: 2211-1247